Emergent Care / Stabilization:
Intestinal malrotation with volvulus in the infant can present with bilious emesis, abdominal distention and/or pain, and hypovolemic and/or septic shock.

Urgent stabilization should include intravenous (IV) fluid resuscitation, early administration of broad-spectrum antibiotics, gastric decompression, imaging, and prompt surgical consultation with the goal of minimizing bowel ischemia.

Older children and adults may have undiagnosed malrotation and can develop acute midgut volvulus at any time, requiring prompt surgical consultation.

Diagnosis Overview:
Malrotation is a term used to describe a spectrum of intestinal rotational anomalies, the most common of which involves abnormal formation and fixation of the intestinal stalk to the abdominal wall, resulting in a higher propensity of developing midgut volvulus, particularly in infants younger than 1 year.

Intestinal malrotation is a congenital anomaly of abnormal embryologic intestinal rotation occurring in approximately 1:500 live births; however, the true incidence is largely unknown as rotational anomalies can remain asymptomatic throughout adult life. The most common anatomic variant includes the cecum lying in the right-upper abdomen, the duodenal-jejunal flexure occurring right of midline, and a narrow mesenteric stalk, which can predispose to midgut volvulus. Ladd bands, thick, fibrous tissue passing from the cecum to the duodenum, may also cause compression and symptoms of obstruction.

Intestinal malrotation has been found to be associated with intestinal atresia, Meckel diverticulum, intussusception, Hirschsprung disease, congenital diaphragmatic hernias, congenital heart disease, and heterotaxy syndrome.

Symptoms and complications of malrotation can present from infancy through adulthood, but studies show that the vast majority present in very early childhood; 50%-70% of cases present within the first month of life and 90% in the first year.

The classic presentation is that of an infant with midgut volvulus, a life-threatening complication of intestinal malrotation resulting in twisting of the narrow mesenteric base leading to bowel obstruction and intestinal ischemia. These infants most commonly present with acute onset of bilious emesis. Late symptoms of ischemic bowel include irritability, abdominal distention, abdominal wall erythema, hematochezia, and signs of hypovolemic and/or septic shock.

Older children or adults may develop chronic midgut volvulus due to incomplete twisting from lymphatic and venous obstruction leading to intermittent abdominal pain, bloating, gastroesophageal reflux, emesis, food intolerance, weight loss, and malabsorption. These symptoms may spontaneously resolve between episodes. Acute volvulus or complete obstruction may present with sudden onset acute abdominal pain, distention, bilious emesis, tachypnea, hematochezia, and signs of shock.

Some studies have shown that chronic symptoms were found in 90% of adults due to the delayed diagnosis of malrotation given its low prevalence in the adult population. Studies have also shown that malrotation can be missed in adults who have undergone previous abdominal imaging or surgeries.